关键词: AIH, autoimmune hepatitis ALT, alanine aminotransferase ANA, antinuclear antibody AST, aminotransferase AZA, azathioprine Autoimmune hepatitis B cells BAFF BAFF, B cell-activating factor Belimumab CR, complete response DCs, dendritic cells EBV, Epstein–Barr virus GGT, gamma-glutamyl transferase LSM, liver stiffness measurements MMF, mycophenolate mofetil SLE, systemic lupus erythematosus TNF, tumour necrosis factor Treatment Tregs, regulatory T cells ULN, upper limit of normal

来  源:   DOI:10.1016/j.jhepr.2020.100123   PDF(Sci-hub)   PDF(Pubmed)

Abstract:
UNASSIGNED: Autoimmune hepatitis (AIH) is a disease of unknown aetiology with a favourable response to immunosuppression. However, in the clinic, it appears that <50% of patients achieve complete response on standard treatment. Serum B cell-activating factor (BAFF) levels are elevated in patients with AIH and are likely to contribute to disease pathogenesis. Given that belimumab, a BAFF inhibitor, has been shown to be effective in other autoimmune diseases, we investigated its use as a third-line add-on treatment option in patients with advanced AIH who did not respond to conventional treatment.
UNASSIGNED: Herein, we report for the first time two patients, a 27-year-old female and a 58-year-old male, both with AIH-related compensated cirrhosis at diagnosis, who were refractory to standard immunosuppressive therapies and received add-on third-line therapy with belimumab.
UNASSIGNED: Both patients achieved a complete response and remained in remission while receiving low-dose corticosteroids. No adverse events related to belimumab and/or disease decompensation were observed.
UNASSIGNED: These preliminary findings indicate belimumab as a promising treatment option for patients with AIH and refractory and advanced liver-related fibrosis.
UNASSIGNED: A small proportion of patients with autoimmune hepatitis (AIH) are refractory to standard treatments; these patients bear the highest probability of developing decompensated cirrhosis and hepatocellular carcinoma because third-line treatment options are not well established. In this case study, we showed that third-line add-on therapy with belimumab, a B cell-activating factor inhibitor, could be an alternative and promising treatment option in patients with advanced AIH who did not respond to conventional treatment.
摘要:
暂无翻译
公众号